Living with Lupus
Systemic Lupus Erythematosus (SLE) is the consequence of inflammation in
the body. It is caused by an attack from your immune system on your own
body.
This is also known as an autoimmune attack – i.e. a self attack.
Under normal circumstances whenever we eat, breathe or drink, millions of
pathogens (bad bacteria, viruses, germs) enter our body. Rather like a radar
system, the immune system is able to ‘scan’ and spot these invaders, and
distinguish them as foreign i.e. not part of your body. It then surrounds and
destroys them. The ability to distinguish the body’s own tissues from these
invaders is critical to the body’s survival.
The attack against the body’s own immune system that occurs in SLE, results
in a wide variety of clinical problems ranging from rashes to convulsions, from
arthritis to mouth ulcers. Any organ or system within the body can be
affected, although some, such as the skin and joints are much more
frequently affected than others. For example, it is rare for the liver or eyes to
be involved.
The vast majority of people who develop lupus are women (around 90%) and
most do so after the onset of periods and before the menopause (between 1550 years).
The American College of Rheumatology have produced a set of classification
criteria, for the diagnosis of Lupus. It is generally accepted that in order to be
diagnosed with Lupus four (or more) of these features should be present.
1. Malar rash – facial rash usually across the bridge of the nose and
forehead – the ‘butterfly rash’(which is named after its shape)
2. Discoid rash – a raised ‘disc like’ rash present on the body at various
sites e.g. over the neck, upper chest and arms.
3. Photosensitivity – sensitivity to the sun which causes rashes and can
cause a flare of your condition
4. Oral ulcers
5. Arthritis – inflammation of the joints
6. Pleuritis – inflammation of the lining of the lungs
7. Pericarditis – inflammation of the lining of the heart
8. Kidney disorder – inflammation of the kidney
9. Neurological problems – for example, seizures (‘fits’ like those seen in
epilepsy), headaches/migraines, psychosis (extremely rare)
10. Blood disorders – anaemia, low white blood cell count (Leucopenia,
lymphopenia or neutropenia), low platelet count (thrombocytopenia) If
levels of platelets are low (thrombocytopenia).
11. Immunological disorders - for example, the presence of autoantibodies (which attack the body’s own tissues). These have some
strange names such as anti-double-stranded (ds) DNA antibodies; antiSm antibodies and anti-phospholipid antibodies.
Common features of Lupus
Lupus is a very strange condition that can affect virtually any organ or system
in the body. Because of this it is very easy to misdiagnosis lupus.
1. Skin rashes
The most obvious and common presentation of lupus is the ‘butterfly rash’,
which is named after its shape as it involves much of the cheeks with a
narrow bridge across the nose. About one third of people develop this. The
skin is usually red; a little raised, and occasionally blisters.
The skin, particularly for people with fair skin, is often sensitive to the sun.
Besides the face, the upper chest, forearms and hands are often affected too.
Sensitivity to the sun means that it is easy for people with lupus to develop
skin rashes when exposed to the sun. During the summer or for those going
on holidays to the beach or hot places in search of a tan; or even driving a car
with the window open, can lead to a lupus rash.
Rashes seem to be caused by inflammation of small blood vessels. This is
called vasculitis. This process is part of the disease process itself, but the
effects are just more visible on the skin compared to the inner organs.
Loss of hair (alopecia) is also linked with skin involvement. This may be mild
and happen generally across the head or in less that 10% of people it may be
more severe and occur in patches or across the head.
2. Swollen joints
Inflammation of the joints, often starting with simple aching on movement, is
another very common feature in early lupus (over 90% of people). Joint
aching (arthralgia) usually occurs in the small joints of the hands and
sometimes the feet. This often progresses to the larger joints, such as the
wrists, elbows, and knees. Aching (arthralgia) may go on to develop into real
swelling of the joints when the term arthritis is used.
3. Lungs and heart
Inflammation of the tissues around the lungs (pleurisy) or heart (pericarditis)
are also recognised early features of lupus. Both organs are surrounded by
thin layers of tissue which may easily become inflamed. These problems
typically give rise to pain in the chest (especially at the end of taking a deep
breath) or shortness of breath. In very rare, more troublesome disease fluid
may collect around the lungs or heart which can interfere with breathing or the
heart beating.
More importantly it is known that lupus patients are far more prone to the
development of atherosclerosis. This is a problem affecting blood vessels. It
is like the way that water pipes may become clogged up and eventually
blocked over the years. The important ‘pipes’ in this case are the blood
vessels in the heart muscle itself. When this blood supply is interfered with,
small portions of the heart muscle tissue may die causing a heart attack.
It is not known why lupus patients have an increased risk of this. The normal
risk factors are:
 Smoking
 Family history of heart attacks or strokes
 High cholesterol levels
 High blood pressure
 Diabetes
It is important that people with lupus regularly have their blood pressure and
cholesterol checked (as it is easy to correct this) and don’t smoke!
4. The kidneys
The kidneys are essential organs in the body. They filter and remove waste
products from the blood into the urine. Approximately one third of people with
lupus have inflammation in their kidneys. If this is not treated then in extreme
cases it can lead to kidney failure. Problems with the kidney are found using
simple urine tests and other investigations (such as a kidney biopsy). It is
rare that someone would be aware that there are any problems until there
was very severe damage.
The urine test is looking for the presence of protein and blood in the urine.
High blood pressure is often linked with kidney problems. This is another
‘silent problem’ that people are not aware of unless it is measured.
5. The blood
Many people suffer from low levels of haemoglobin (the pigment inside red
blood cells that carries oxygen around the body), white blood cells and
platelets.
A low level of haemoglobin can lead to anaemia, which can make people feel
very tired (fatigued).
The white blood cells are essential to the normal defence of the body against
bacteria, viruses and fungi. Around 40% of people with lupus have low levels
of white blood cells in general, and 80% have a low level of lymphocytes,
which is one type of white blood cell. This is called lymphocytopaenia. This
means that people with lupus are more susceptible to infection and can take
longer to recover.
Platelets are the cells involved in producing a blood clot when we cut
ourselves. Without sufficient number of these cells, there is a tendency to
bleed, sometimes quite severely and bruise more easily. This is called
thrombocytopaenia.
6. Other problems
A group of features commonly found early in the course of lupus, and often
occurring together, include:
 Fatigue – severe tiredness that doesn’t go away with rest
 Fever – can be very significant (temperature exceeding 39oC)
 Swollen lymph glands
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Loss of appetite
Sickness